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Referral and warning signs
When to refer
Referral to specialized Haematology/Oncology centre for ALL suspected cases for biopsy, diagnosis, staging, and treatment.
Red flags
- Progressive or persistent lymphadenopathy
- 'B' symptoms (fever, night sweats, weight loss)
- Rapidly enlarging nodes/masses
- Signs of organ infiltration or bulky disease (e.g., SVC obstruction, cytopenias)
- Associated autoimmune phenomena
Signs and symptoms
- Painless, progressive lymphadenopathy.
- 'B' Symptoms (more common in aggressive NHL): Unexplained fever (>38°C), Drenching night sweats, Unexplained weight loss (>10% in 6 months).
- Fatigue, malaise.
- Symptoms related to extranodal involvement: GI tract (pain, bleeding, obstruction), Bone marrow (cytopenias), CNS (headache, seizures, deficits), Skin (plaques, nodules, ulcers), Spleen/Liver enlargement, Testicular mass.
- Symptoms related to bulky disease (e.g., SVC obstruction).
Risk factors
- Immunodeficiency (HIV/AIDS - major risk factor; Post-transplant).
- Autoimmune diseases (Sjogren's, RA).
- Certain infections (EBV, HTLV-1, H. pylori, HCV).
- Environmental exposures (pesticides - controversial).
- Family history (rare).
- Increasing age.
Diagnosis
Clinical assessment
Suspect in patient with persistent/progressive lymphadenopathy, +/- B symptoms, +/- extranodal signs. Differentiate from reactive nodes, Hodgkin's, TB, metastatic cancer.
Investigations
- Excisional Lymph Node Biopsy: Essential for diagnosis and subtyping. Provides architecture. FNA inadequate.
- Immunohistochemistry and Flow Cytometry: Determine lineage (B/T cell) and subtype markers.
- Cytogenetics / Molecular Studies: Identify translocations/mutations.
- Staging Investigations: CT scan (Neck, Chest, Abdomen, Pelvis), PET-CT scan (often preferred), Bone Marrow Aspiration and Biopsy, Lumbar Puncture (if CNS symptoms/high-risk subtype).
- Blood tests: CBC, LDH, Uric acid, LFTs, RFTs, Viral serology (HIV, HBV, HCV, EBV).
Diagnostic criteria
Histopathological confirmation from biopsy, classified according to WHO criteria.
Management
Non-pharmacological management
- Referral to specialized Haematology/Oncology centre.
- Management depends on subtype and stage.
- Indolent NHL: Watchful waiting (asymptomatic) vs Treatment (symptoms/bulk/cytopenias).
- Aggressive NHL: Immediate curative-intent treatment.
- Supportive care: Infection management, tumour lysis prophylaxis, psychosocial support.
Pharmacological treatment
- Treatment varies widely:
- - Aggressive B-cell NHL (e.g., DLBCL): Immuno-chemotherapy (R-CHOP standard).
- - Burkitt's Lymphoma: Intensive short-course chemo + CNS prophylaxis.
- - Indolent B-cell NHL: Rituximab +/- chemo (R-CVP, R-Bendamustine). Maintenance Rituximab.
- - T-cell Lymphomas: Often CHOP-like regimens.
- Radiotherapy: Localized disease or consolidation/palliation.
- Stem Cell Transplantation: Relapsed/refractory aggressive NHL.
Monitoring
Monitor treatment response (imaging). Monitor toxicity. Long-term follow-up for relapse and late effects.
Complications
- Complications from tumour bulk (obstruction, organ dysfunction, CNS involvement).
- Bone marrow failure.
- Tumour Lysis Syndrome.
- Infections.
- Treatment toxicity.
- Transformation (indolent to aggressive).
- Relapse.
Prevention
- Treat underlying causative infections (H. pylori, HCV).
- Manage immunosuppression (ART for HIV).
Patient education
- Explain NHL diagnosis, subtype, stage.
- Discuss treatment plan and goals.
- Potential side effects.
- Importance of adherence and monitoring.
- Infection precautions.
- Long-term follow-up plan.
- Prognosis varies greatly by subtype.
Uganda-specific notes
High incidence of aggressive NHL, particularly associated with HIV and EBV. Diagnosis requires reliable pathology/immunohistochemistry. Access to standard chemo-immunotherapy (Rituximab), advanced imaging (PET-CT), and stem cell transplant is limited.
References
- Uganda Clinical Guidelines (UCG) 2023
Chapter 12, Section 12.3.2
- Uganda Cancer Institute Treatment Guidelines
(Specific protocols)